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大片状肺泡出血: Wegener 肉芽肿的一种罕见致死性并发症——罕见病例报告。

Massive alveolar haemorrhage: a rare life threatening complication of Wegener's granulomatosis-report of a rare case.

机构信息

Department of Radiology and Imaging, Nizams Institute of Medical Sciences, Punjagutta, Hyderabad 500082, India.

出版信息

Quant Imaging Med Surg. 2014 Dec;4(6):509-11. doi: 10.3978/j.issn.2223-4292.2014.07.03.

DOI:10.3978/j.issn.2223-4292.2014.07.03
PMID:25525588
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4256243/
Abstract

Massive diffuse alveolar haemorrhage (DAH) is an unusual, frequently life threatening and still treatable complication of Wegener's granulomatosis (WG) associated with mortality in more than 66% of cases. DAH is a syndrome that can be a manifestation of variety of disorders. WG, a multisystem necrotising granulomatous vasculitis should always be considered as a possible etiological factor in these patients with or without involvement of other systems, as an early diagnosis and aggressive management with cytotoxic drugs offer best chances of survival in these patients. We report a rare case of 45-year-old woman who presented with massive hemoptysis in accident and emergency department and subsequently diagnosed as WG, and discuss the differential diagnosis and review the literature of this rare complication of WG. Approximately 41 cases have been reported so far of this rare complication.

摘要

弥漫性肺泡出血(DAH)是韦格纳肉芽肿(WG)的一种罕见、常危及生命但仍可治疗的并发症,其死亡率超过 66%。DAH 是一种综合征,可由多种疾病引起。WG 是一种多系统坏死性肉芽肿性血管炎,在这些有或无其他系统受累的患者中,均应始终考虑将其作为可能的病因,因为早期诊断和使用细胞毒性药物积极治疗可最大程度提高这些患者的生存率。我们报告了一例罕见的 45 岁女性病例,她因大咯血到急诊就诊,随后被诊断为 WG,并讨论了这种罕见并发症的鉴别诊断和文献复习。到目前为止,大约有 41 例这种罕见并发症的报道。

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本文引用的文献

1
Wegener's granulomatosis in a middle-aged woman presenting with dyspnea, rash, hemoptysis and recurrent eye complaints: a case report.一名中年女性出现呼吸困难、皮疹、咯血及反复眼部不适的韦格纳肉芽肿病:病例报告
J Med Case Rep. 2012 Oct 3;6:335. doi: 10.1186/1752-1947-6-335.
2
Antineutrophil cytoplasmic antibody-associated vasculitis complicated with diffuse alveolar hemorrhage: a study of 12 cases.抗中性粒细胞胞浆抗体相关性血管炎并发弥漫性肺泡出血:12 例研究。
J Clin Rheumatol. 2009 Oct;15(7):341-4. doi: 10.1097/RHU.0b013e3181b59581.
3
Diffuse alveolar hemorrhage: diagnosing it and finding the cause.弥漫性肺泡出血:诊断及病因查找
Cleve Clin J Med. 2008 Apr;75(4):258, 260, 264-5 passim. doi: 10.3949/ccjm.75.4.258.
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Pulmonary manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis.抗中性粒细胞胞浆抗体(ANCA)阳性血管炎的肺部表现。
Rheumatology (Oxford). 2006 Mar;45(3):261-8. doi: 10.1093/rheumatology/kei217. Epub 2006 Jan 17.
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Small vessel vasculitis of the lung.肺小血管炎
Thorax. 2000 Jun;55(6):502-10. doi: 10.1136/thorax.55.6.502.